Cerebrovascular disease hospitalizations following emergency department headache visits: A nested case-control study.

BACKGROUND: Delayed diagnosis of cerebrovascular disease (CVD) among patients can result in substantial harm. If diagnostic process failures can be identified at emergency department (ED) visits that precede CVD hospitalization, interventions to improve diagnostic accuracy can be developed. METHODS: We conducted a nested case-control study using a cohort of adult ED patients discharged from a single medical center with a benign headache diagnosis from October 1, 2015 to March 31, 2018. Hospitalizations for CVD within 1 year of index ED visit were identified using a regional health information exchange. Patients with subsequent CVD hospitalization (cases) were individually matched to patients without subsequent hospitalization (controls) using patient age and visit date. Demographic, clinical, and ED process characteristics were assessed via detailed chart review. McNemar's test for categorical and paired t-test for continuous variables were used with statistical significance set at ≤0.05. RESULTS: Of the 9157 patients with ED headache visits, 57 (0.6%, 95% confidence interval [CI] = 0.5-0.8) had a subsequent CVD hospitalization. Median time from ED visit to hospitalization was 107 days. In 25 patients (43.9%, 25/57) the CVD hospitalization and the index ED visit were at different hospitals. Fifty-three cases and 53 matched controls were included in the final study analysis. Cases and controls had similar baseline demographic and headache characteristics. Cases more often had a history of stroke (32.1% vs. 13.2%, p = 0.02) and neurosurgery (13.2% vs. 1.9%, p = 0.03) prior to the index ED visit. Cases more often had less than two components of the neurologic examination documented (30.2% vs. 11.3%, p = 0.03). CONCLUSION: We found that 0.6% of patients with an ED headache visit had subsequent CVD hospitalization, often at another medical center. ED visits for headache complaints among patients with prior stroke or neurosurgical procedures may be important opportunities for CVD prevention. Documented neurologic examinations were poorer among cases, which may represent an opportunity for ED process improvement.

Trends and Characterization of Academic Emergency Department Patient Visits: A Five-year Review.

OBJECTIVES: To meet the unique comparative data needs of academic emergency departments (EDs), we describe the recent 5-year national and regional trends for adult emergency patients' characteristics and operational parameters at academic emergency medical centers. METHODS: Data collected from the recent 5-year period academic year (AY) 2012 through AY 2016 of the Academy of Administrators in Academic Emergency Medicine (AAAEM) and the Association of Academic Chairs of Emergency Medicine (AACEM) Academic Emergency Medicine Benchmarking Survey were analyzed for trends in 1) ED volumes and modes of arrival, 2) triage acuity level, 3) trends in ED professional fee billing, and 4) disposition patterns of ED patients including admission rates and walkouts. The AY spanned the 12-month period of July 1 through June 30. Only primary academic or academic affiliate hospitals data were included. Community and freestanding affiliated EDs were excluded. Institutional-specific data were stratified into four cohorts based on the following annual ED visit volumes: under 40,000, 40,000 to 60,000, 60,000 to 80,000, and over 80,000. Triage acuity levels were based on the Emergency Severity Index (ESI). Professional fee billing was analyzed specifically for CPT codes 99284, 99285, and 99291 (critical care). Left without being seen (LWBS), defined as leaving before a screening examine by a licensed medical provider, and screened and left (SAL), i.e., patients who were screened by a provider, but left before definitive evaluation and management, were similarly evaluated. Total walkouts were defined as the sum of LWBS and SAL. RESULTS: Forty-four primary academic and academic affiliate sites completed the survey for all 5 years. The mean annual patient census increased 13.4% over the study period, with the majority (80%) of sites experiencing volume growth. Acuity/illness severity, measured as ESI 1 and 2, and CPT codes 99284, 99295, and 99291 increased an aggregate 18.2 and 8.4%, respectively. Large-volume hospitals (>60,000-80,000, >80,000) admissions increased by 15 and 21.6%, respectively, primarily due to surge in 2016. Overall emergency medical services (EMS) arrivals increased 7.3% although admissions from EMS remained relatively stable. LWBS rates decreased 19.5%, but total walkouts did not appear to change. CONCLUSION: With a focus on larger academic institutions, differences were noted in the overall increases in volume and acuity. In this survey, participating institutions experienced increased volumes of patients with seemingly higher illness severity. While inroads have been made in LWBS rates, there has not been an overall decrease in total walkouts. The data reported here differed in many aspects compared to other benchmark surveys.

Emergency Department Utilization Among the Uninsured During Insurance Expansion in Maryland.

STUDY OBJECTIVE: We analyzed the effect of insurance expansion on emergency department (ED) utilization among the uninsured in Maryland, which expanded Medicaid eligibility and created health insurance exchanges in 2014. METHODS: This was a retrospective analysis of statewide administrative claims for July 2012 to December 2015. We used coarsened exact matching to pair uninsured and insured (Medicaid, Medicare, commercial, and other) adult Maryland residents who visited an ED or were hospitalized at baseline (July 2012 to December 2013). We compared ED utilization between these groups after insurance expansion (January 2014 to December 2015), using a difference-in-differences quasi-experimental design. Nonreturning patients from the baseline period were included in the post-insurance expansion rates as having zero visits. RESULTS: Matching yielded 178,381 pairs. In the 12 months before insurance expansion, the baseline uninsured group visited the ED at a rate of 26.1 per 100 patient-quarters versus 28.2 among the insured group (relative rate=0.93). In the 24 months after insurance expansion, 45% of the baseline uninsured returned to an ED, of whom 33% returned uninsured, 40% returned with Medicaid, and 21% returned with commercial insurance. After insurance expansion, with 55% of patients in each group not returning, the ED visit rate for both the baseline uninsured and insured groups was 15.9 per 100 patient-quarters (relative rate=1.00). This 8% relative increase from baseline in ED visits among the uninsured group was driven primarily by increases in higher-acuity visits. Uninsured patients from high-poverty zip codes (N=34,964 pairs) increased their ED utilization by 15% after insurance expansion, whereas baseline uninsured patients with no comorbidities (N=94,330 pairs) showed a 3% decrease. CONCLUSION: Insurance expansion in Maryland was associated with a modest relative increase in ED visits among the uninsured, driven by increases in higher-acuity visits. It remains unclear whether insurance coverage helped the uninsured address their unmet medical needs.

Implementing standardized, inter-unit communication in an international setting: handoff of patients from emergency medicine to internal medicine.

Standardized handoffs may reduce communication errors, but research on handoff in community and international settings is lacking. Our study at a community hospital in the United Arab Emirates characterizes existing handoff practices for admitted patients from emergency medicine (EM) to internal medicine (IM), develops a standardized handoff tool, and assesses its impact on communication and physician perceptions. EM physicians completed a survey regarding handoff practices and expectations. Trained observers utilized a checklist based on the Systems Engineering Initiative for Patient Safety model to observe 40 handoffs. EM and IM physicians collaboratively developed a written tool encouraging bedside handoff of admitted patients. After the intervention, surveys of EM physicians and 40 observations were subsequently repeated. 77.5% of initial observed handoffs occurred face-to-face, with 42.5% at bedside, and in four different languages. Most survey respondents considered face-to-face handoff ideal. Respondents noted 9-13 patients suffering harm due to handoff in the prior month. After handoff tool implementation, 97.5% of observed handoffs occurred face-to-face (versus 77.5%, p = 0.014), with 82.5% at bedside (versus 42.5%, p < 0.001), and all in English. Handoff was streamlined from 7 possible pathways to 3. Most post-intervention survey respondents reported improved workflow (77.8%) and safety (83.3%); none reported patient harm. Respondents and observers noted reduced inefficiency (p < 0.05). Our standardized tool increased face-to-face and bedside handoff, positively impacted workflow, and increased perceptions of safety by EM physicians in an international, non-academic setting. Our three-step approach can be applied towards developing standardized, context-specific inter-specialty handoff in a variety of settings.

Analgesic Access for Acute Abdominal Pain in the Emergency Department Among Racial/Ethnic Minority Patients: A Nationwide Examination.

BACKGROUND: Prior studies of acute abdominal pain provide conflicting data regarding the presence of racial/ethnic disparities in the emergency department (ED). OBJECTIVE: To evaluate race/ethnicity-based differences in ED analgesic pain management among a national sample of adult patients with acute abdominal pain based on a uniform definition. RESEARCH DESIGN/SUBJECTS/MEASURES: The 2006-2010 CDC-NHAMCS data were retrospectively queried for patients 18 years and above presenting with a primary diagnosis of nontraumatic acute abdominal pain as defined by the American Association for the Surgery of Trauma. Independent predictors of analgesic/narcotic-specific analgesic receipt were determined. Risk-adjusted multivariable analyses were then performed to determine associations between race/ethnicity and analgesic receipt. Stratified analyses considered risk-adjusted differences by the level of patient-reported pain on presentation. Secondary outcomes included: prolonged ED-LOS (>6 h), ED wait time, number of diagnostic tests, and subsequent inpatient admission. RESULTS: A total of 6710 ED visits were included: 61.2% (n=4106) non-Hispanic white, 20.1% (n=1352) non-Hispanic black, 14.0% (n=939) Hispanic, and 4.7% (n=313) other racial/ethnic group patients. Relative to non-Hispanic white patients, non-Hispanic black patients and patients of other races/ethnicities had 22%-30% lower risk-adjusted odds of analgesic receipt [OR (95% CI)=0.78 (0.67-0.90); 0.70 (0.56-0.88)]. They had 17%-30% lower risk-adjusted odds of narcotic analgesic receipt (P<0.05). Associations persisted for patients with moderate-severe pain but were insignificant for mild pain presentations. When stratified by the proportion of minority patients treated and the proportion of patients reporting severe pain, discrepancies in analgesic receipt were concentrated in hospitals treating the largest percentages of both. CONCLUSIONS: Analysis of 5 years of CDC-NHAMCS data corroborates the presence of racial/ethnic disparities in ED management of pain on a national scale. On the basis of a uniform definition, the results establish the need for concerted quality-improvement efforts to ensure that all patients, regardless of race/ethnicity, receive optimal access to pain relief.

Breast cancer in female survivors of Wilms tumor: a report from the national Wilms tumor late effects study.

BACKGROUND: The standard treatment of pulmonary metastases in patients with Wilms tumor (WT) includes 12-gray radiotherapy (RT) to the entire chest. To the authors' knowledge, the risk of breast cancer (BC) in a large cohort of female survivors of WT has not previously been reported. METHODS: A total of 2492 female participants in National Wilms Tumor Studies 1 through 4 (1969-1995) were followed from age 15 years through the middle of 2013 for incident BC. The median age at the time of last contact was 27.3 years. The authors calculated cumulative risk at age 40 years (CR40), hazard ratios (HR) by Cox regression, standardized incidence ratios (SIRs) relative to US population rates, and 95% confidence intervals (95% CIs). RESULTS: The numbers of survivors with invasive BC divided by the numbers at risk were 16 of 369 (CR40, 14.8% [95% CI, 8.7-24.5]) for women who received chest RT for metastatic WT, 10 of 894 (CR40, 3.1% [95% CI, 1.3-7.41]) for those who received only abdominal RT, and 2 of 1229 (CR40, 0.3% [95% CI, 0.0-2.3]) for those who received no RT. The SIRs for these 3 groups were 27.6 (95% CI, 16.1-44.2) based on 5010 person-years (PY) of follow-up, 6.0 (95% CI, 2.9-11.0) based on 13,185 PY of follow-up, and 2.2 (95% CI, 0.3-7.8) based on 13,560 PY of follow-up, respectively. The risk was high regardless of the use of chest RT among women diagnosed with WT at age ≥10 years, with 9 of 90 women developing BC (CR40, 13.5% [95% CI, 5.6-30.6]; SIR, 23.6 [95% CI, 10.8-44.8] [PY, 1463]). CONCLUSIONS: Female survivors of WT who were treated with chest RT had a high risk of developing early BC, with nearly 15% developing invasive disease by age 40 years. Current guidelines that recommend screening only those survivors who received ≥20 Gy of RT to the chest might be reevaluated.

Resident to resident handoffs in the emergency department: an observational study.

BACKGROUND: Despite patient handoffs being well recognized as a potentially dangerous time in the care of patients in the emergency department (ED), there is no established standard and little supporting research on how to optimize the process. Minimizing handoff risks is particularly important at teaching hospitals, where residents often provide the majority of patient handoffs. OBJECTIVE: Our aim was to identify hazards to patient safety and barriers to efficiency related to resident handoffs in the ED. METHODS: An observational study was completed using the Systems Engineering Initiative for Patient Safety model to assess the safety and efficiency of resident handoffs. Thirty resident handoffs were observed with residents in emergency medicine over 16 weeks. RESULTS: Residents were interrupted, on average, every 8.5 min. The most common deficit in relaying the plan of care strategy was failing to relay medications administered (32%). In addition, there were ambiguities related to medication administration, such as when the medication was next due or why a medication was chosen, in 56% of handoffs observed. Ninety percent of residents observed took handwritten notes. A small percentage (11%) also completed free texted computer progress notes. Ten percent of residents took no notes. CONCLUSIONS: The existing system allows for a clear summary of the patient's visit. Two major deficits-frequent interruptions and inconsistent communication regarding medications administered-were noted. There is inconsistency in how information is recorded at the time of handoff. Future studies should focus on handoff improvement and error reduction.

Pulmonary disease after treatment for Wilms tumor: a report from the national wilms tumor long-term follow-up study.

PURPOSE: This study was undertaken to evaluate the incidence of pulmonary disease among patients treated with radiation therapy (RT) for pulmonary metastases (PM) from Wilms tumor (WT). PATIENTS AND METHODS: We reviewed records of 6,449 patients treated on National Wilms Tumor Studies-1, -2, -3, and -4 whose flow sheets or annual status reports documented one of several pulmonary conditions. Cases were fully evaluable if pulmonary function test (PFT) results were available, pulmonary fibrosis was identified on a chest radiograph or was listed as the primary or a contributing factor to death. Partially evaluable cases were those for whom PFT results could not be obtained. We evaluated the relationship between RT factors and the occurrence of pulmonary disease using hazard ratios (HRs) and cumulative incidence, treating death as a competing risk. RESULTS: Sixty-four fully evaluable and 16 partially evaluable cases of pulmonary disease were identified. The cumulative incidence of pulmonary disease at 15 years since WT diagnosis was 4.0% (95% confidence interval [CI] 2.6-5.4%) among fully evaluable and 4.8% (95% CI 3.3-6.4%) among fully and partially evaluable patients who received lung RT for PM at initial diagnosis. Rates of pulmonary disease were substantially higher among those who received lung RT for PM present at initial diagnosis or relapse compared to those who received no RT or only abdominal RT (HR 30.2, 95% CI 16.9-53.9). CONCLUSION: The risk of pulmonary disease must be considered in evaluating the risk:benefit ratio of lung RT for the management of PM from WT.

Treatments and outcomes for end-stage renal disease following Wilms tumor.

BACKGROUND: Little is known about treatment outcomes for children who have end-stage renal disease (ESRD) after treatment for Wilms tumor (WT). METHODS: Time-to-transplant, graft failure, and survival outcomes were examined for 173 children enrolled on the National Wilms Tumor Study who developed ESRD. RESULTS: Fifty-five patients whose ESRD resulted from progressive bilateral WT (PBWT) experienced high early mortality from WT that limited their opportunity for transplant (47% at 5 years) and survival (44% at 10 years) in comparison to population controls. The 118 patients whose ESRD was due to other causes (termed "chronic kidney disease"), many of whom had WT-associated congenital anomalies, had transplant (77% at 5 years) and survival (73% at 10 years) outcomes no worse than those for population controls. Graft failure following transplant was comparable for the two groups. Minority children had twice the median time to transplant as non-Hispanic whites and twice the mortality rates, also reflecting population trends. CONCLUSIONS: In view of the continuing high mortality in patients with ESRD, and the dramatic improvement in outlook following kidney transplantation, re-evaluation of current guidelines for a 2-year delay in transplant following WT treatment may be warranted.

Risk factors for end stage renal disease in non-WT1-syndromic Wilms tumor.

PURPOSE: We assessed risk factors for end stage renal disease in patients with Wilms tumor without known WT1 related syndromes. We hypothesized that patients with characteristics suggestive of a WT1 etiology (early onset, stromal predominant histology, intralobar nephrogenic rests) would have a higher risk of end stage renal disease due to chronic renal failure. We predicted a high risk of end stage renal disease due to progressive bilateral Wilms tumor in patients with metachronous bilateral disease. MATERIALS AND METHODS: End stage renal disease was ascertained in 100 of 7,950 nonsyndromic patients enrolled in a National Wilms Tumor Study during 1969 to 2002. Risk factors were evaluated with cumulative incidence curves and proportional hazard regressions. RESULTS: The cumulative incidence of end stage renal disease due to chronic renal failure 20 years after Wilms tumor diagnosis was 0.7%. For end stage renal disease due to progressive bilateral Wilms tumor the incidence was 4.0% at 3 years after diagnosis in patients with synchronous bilateral Wilms tumor and 19.3% in those with metachronous bilateral Wilms tumor. For end stage renal disease due to chronic renal failure stromal predominant histology had a HR of 6.4 relative to mixed (95% CI 3.4, 11.9; p<0.001), intralobar rests had a HR of 5.9 relative to no rests (95% CI 2.0, 17.3; p=0.001), and Wilms tumor diagnosis at less than 24 months had a HR of 1.7 relative to 24 to 48 months and 2.8 relative to greater than 48 months (p=0.003 for trend). CONCLUSIONS: Metachronous bilateral Wilms tumor is associated with high rates of end stage renal disease due to surgery for progressive Wilms tumor. Characteristics associated with a WT1 etiology markedly increased the risk of end stage renal disease due to chronic renal failure despite the low risk in non-WT1 syndromic cases overall.

The management of synchronous bilateral Wilms tumor: a report from the National Wilms Tumor Study Group.

OBJECTIVE: To provide guidelines for future trials, we reviewed the outcomes of children with synchronous bilateral Wilms tumors (BWT) treated on National Wilms Tumor Study-4 (NWTS-4). METHODS: NWTS-4 enrolled 3335 patients including 188 patients with BWT (5.6%). Treatment and outcome data were collected. RESULTS: Among 188 BWT patients registered with NWTS-4, 195 kidneys in 123 patients had initial open biopsy, 44 kidneys in 31 patients had needle biopsies. Although pre-resection chemotherapy was recommended, 87 kidneys in 83 patients were managed with primary resection: Complete nephrectomy 48 in 48 patients, 31 partial/wedge nephrectomies in 27 patients, enucleations 8 in 8 patients. No initial surgery was performed in 45 kidneys in 43 patients, 5 kidneys in 3 patients not coded. Anaplasia was diagnosed after completion of the initial course of chemotherapy in 14 patients (initial surgical procedure: 9 open biopsies, 4 needle biopsies, 1 partial nephrectomy). The average number of days from the start of chemotherapy to diagnosis of anaplasia was 390 (range 44-1925 days). Relapse or progression of disease occurred in 54 children. End stage renal failure occurred in 23 children, 6 of whom had bilateral nephrectomies. The 8 year event free survival for BWT with favorable histology was 74%, and overall survival was 89%; whereas the event free survival for BWT with unfavorable histology was 40%, overall survival was 45%. CONCLUSION: The current analysis of patients with BWT treated on NWTS-4 shows that preservation of renal parenchyma is possible in many patients after initial preoperative chemotherapy. The incidence of end-stage renal disease remains significantly higher in children with BWT. Future studies are warranted to address the need for earlier biopsy in nonresponsive tumors and earlier definitive surgery to recognize unfavorable histology in these high-risk patients.

Pregnancy outcome after treatment for Wilms tumor: a report from the national Wilms tumor long-term follow-up study.

PURPOSE: This study was undertaken to evaluate the effect of prior treatment with radiation therapy or chemotherapy for unilateral Wilms tumor (WT) diagnosed during childhood on pregnancy complications, birth weight, and the frequency of congenital malformations in live-born offspring. PATIENTS AND METHODS: We reviewed pregnancy outcomes among female survivors and partners of male survivors of WT treated on National Wilms Tumor Studies 1, 2, 3, and 4 by using a maternal questionnaire and a review of both maternal and offspring medical records. RESULTS: We received reports of 1,021 pregnancies with duration of 20 weeks or longer, including 955 live-born singletons, for whom 700 sets of maternal and offspring medical records were reviewed. Rates of hypertension complicating pregnancy (International Classification of Diseases [ICD] code 642), early or threatened labor (ICD-644) and malposition of the fetus (ICD-652) increased with increasing radiation dose in female patients. The percentages of offspring weighing less than 2,500 g at birth and of those having less than 37 weeks of gestation also increased with dose. There was no significant trend with radiation dose in the number of congenital anomalies recorded in offspring of female patients. CONCLUSION: Women who receive flank radiation therapy as part of the treatment for unilateral WT are at increased risk of hypertension complicating pregnancy, fetal malposition, and premature labor. The offspring of these women are at risk for low birth weight and premature (ie, < 37 weeks gestation) birth. These risks must be considered in the obstetrical management of female survivors of WT.

Secondary malignant neoplasms after Wilms tumor: an international collaborative study.

A combined cohort of 8,884 North American, 2,893 British and 1,574 Nordic subjects with Wilms tumor (WT) diagnosed before 15 years of age during 1960-2004 was established to determine the risk of secondary malignant neoplasms (SMN). After 169,641 person-years (PY) of observation through 2005, 174 solid tumors (exclusive of basal cell carcinomas) and 28 leukemias were ascertained in 195 subjects. Median survival time after a solid SMN diagnosis 5 years or more from WT was 11 years; it was 10 months for all leukemia. Age-specific incidence of secondary solid tumors increased from approximately 1 case per 1,000 PY at age 15 to 5 cases per 1,000 PY at age 40. The cumulative incidence of solid tumors at age 40 for subjects who survived free of SMNs to age 15 was 6.7%. Leukemia risk, by contrast, was highest during the first 5 years after WT diagnosis. Standardized incidence ratios (SIRs) for solid tumors and leukemias were 5.1 and 5.0, respectively. Results for solid tumors for the 3 geographic areas were remarkably consistent; statistical tests for differences in incidence rates and SIRs were all negative. Age-specific incidence rates and SIRs for solid tumors were lower for patients whose WT was diagnosed after 1980, although the trends with decade of diagnosis were not statistically significant. Incidence rates and SIRs for leukemia were highest among those diagnosed after 1990 (p-trend = 0.003). These trends may reflect the decreasing use of radiation therapy and increasing intensity of chemotherapy in modern protocols for treatment of WT.

Portal hypertension in children with Wilms' tumor: a report from the National Wilms' Tumor Study Group.

PURPOSE: This analysis was undertaken to determine the cumulative risk of and risk factors for portal hypertension (PHTN) in patients with Wilms' tumor (WT). METHODS AND MATERIALS: Medical records were reviewed to identify cases of PHTN identified with late liver/spleen/gastric toxicities in a cohort of 5,195 patients treated with National Wilms' Tumor Studies (NWTS) protocols 1 to 4. A nested case control study (5 controls/case) was conducted to determine relationships among doxorubicin, radiation therapy (RT) dose to the liver, patient gender, and PHTN. Conditional logistic regression was used to estimate adjusted hazard ratios (HR) of PHTN associated with these factors. RESULTS: Cumulative risk of PHTN at 6 years from WT diagnosis was 0.7% for patients with right-sided tumors vs. 0.1% for those with left-sided tumors (p = 0.002). Seventeen of 19 cases were evaluable for RT. The majority of cases (16/17 [94%]) received right-flank RT either alone or as part of whole-abdomen RT and received >15 Gy to the liver. Fifteen of 17 (88%) patients received a higher dose to the liver than they would have with modern WT protocols. Controlling for RT dose, the HR was 3.0 for patients who received doxorubicin (p = 0.32) and 2.8 for females (p = 0.15). Controlling for doxorubicin, the 95% lower confidence bound on the HR associating PHTN with a minimum liver RT dose of >15 Gy vs. 15 Gy vs. 15 Gy) and the development of PHTN among WT patients.

End stage renal disease in patients with Wilms tumor: results from the National Wilms Tumor Study Group and the United States Renal Data System.

PURPOSE: We sought to assess accurately the full spectrum of end stage renal disease (ESRD) in Wilms tumor survivors by combining the unique resources of the National Wilms Tumor Study Group (NWTSG) and the United States Renal Data System (USRDS), and to confirm preliminary reports of an increased incidence of ESRD in patients with the Wilms tumor-aniridia syndrome (WAGR). MATERIALS AND METHODS: ESRD was ascertained in 5,910 patients enrolled in NWTSG studies during 1969 to 1994 by record linkage to USRDS and by direct followup. Cumulative ESRD incidence was estimated accounting for intercurrent mortality. RESULTS: Of 115 cases of ESRD 10 (9%) were ascertained by the NWTSG alone, 13 (11%) by USRDS alone and 92 (80%) by both. Cumulative incidence of ESRD at 20 years from diagnosis of unilateral Wilms tumor was 74% for 17 patients with the Denys-Drash syndrome, 36% for 37 patients with WAGR, 7% for 125 male patients with hypospadias or cryptorchidism (genitourinary [GU] anomalies) and 0.6% for 5,347 patients with none of these conditions. The incidence of ESRD after diagnosis of bilateral Wilms tumor was 50% for the Denys-Drash syndrome (6 patients), 90% for WAGR (10), 25% for GU anomaly (25) and 12% for other (409). ESRD in patients with WAGR or GU anomalies tended to occur relatively late, often during or after adolescence. CONCLUSIONS: The risk of ESRD is remarkably low for the majority of patients with Wilms tumor. However, those with WAGR or associated GU anomalies are at higher risk and should be screened indefinitely to facilitate prospective treatment of impaired renal function.